Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is the second most prevalent type of peripheral T-cell lymphoma. Patients with AITL commonly present with systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly, and cutaneous manifestations, which are observed in 50-80% of cases. In the lymph node, AITL is characterized by an atypical expansion of follicular T-helper cells that express germinal center markers and co-stimulate B-cells in the tumor microenvironment. Consequently, the neoplastic T lymphocytes are often associated with Epstein-Barr virus (EBV)-positive B-cells as well as clonally restricted B lymphoid and plasma cell proliferations.  

We describe a 72 year old male with a history of stage III AITL who presented with tender violaceous nodules on the abdomen and thigh, asymptomatic rash on the dorsal hands, and a nontender nodule on the right ulnar forearm.  A subsequent biopsy from both the anterior thigh and ulna revealed dense Kappa restricted plasma cell aggregates and an atypical nodular lymphoid infiltrate with a predominance of B lymphocytes as well as atypical T lymphocytes involving the deep dermis and subcutaneous fat. Epstein Barr Virus Encoding Region by In Situ Hybridization (EBER-ISH) was positive in the B lymphocytes. Molecular studies were positive for both a clonal T cell and a clonal B cell population. Given the patient’s clinical history, a diagnosis of recurrent AITL was rendered. Despite treatment, the patient expired shortly thereafter.